Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston, 77030-4009, USA.
Arch Pathol Lab Med. 2011 Dec;135(12):1597-600. doi: 10.5858/arpa.2010-0554-CR.
Rosai-Dorfman disease (RDD) is characterized histologically by a dense histiocytic infiltrate with emperipolesis and associated lymphocytes, plasma cells, and neutrophils. Eosinophils are not commonly associated. We report a patient with initial thymus and pituitary gland involvement by RDD, who later developed papules on the groin and axilla. Skin biopsies showed admixed histiocytic infiltrates (lymphocytes, neutrophils, and plasma cells) without emperipolesis. A prominent eosinophilic infiltrate was also observed, a feature not, to our knowledge, previously reported. Immunohistochemistry revealed positivity for CD68 (most cells) and S100 protein (scattered cells) and was negative for anti-CD1a. The diagnosis of RDD was established in the clinical context after comparison with the thymic and pituitary lesions (similar histologic features, albeit with fewer eosinophils, and immunohistochemical profiles). We present the first case, to our knowledge, of multicentric RDD with cutaneous involvement and associated prominent eosinophilic infiltrate. Thus, RDD should be included in the differential diagnosis of mononuclear infiltrates containing eosinophils.
罗道尔夫-多夫曼病(RDD)的组织学特征是密集的组织细胞浸润,伴有吞噬现象,并伴有淋巴细胞、浆细胞和中性粒细胞。嗜酸性粒细胞通常不与之相关。我们报告了一例最初累及胸腺和垂体的 RDD 患者,随后在腹股沟和腋窝出现丘疹。皮肤活检显示混合性组织细胞浸润(淋巴细胞、中性粒细胞和浆细胞),无吞噬现象。还观察到明显的嗜酸性粒细胞浸润,据我们所知,这一特征以前没有报道过。免疫组化显示 CD68(大多数细胞)和 S100 蛋白(散在细胞)阳性,抗 CD1a 阴性。在与胸腺和垂体病变(相似的组织学特征,尽管嗜酸性粒细胞较少,免疫组化特征)进行比较后,在临床背景下确立了 RDD 的诊断。我们报告了首例已知的多中心 RDD 伴皮肤受累和相关的显著嗜酸性粒细胞浸润。因此,RDD 应纳入含有嗜酸性粒细胞的单核细胞浸润的鉴别诊断。